AB0635 Central Nervous System Involvement and Mimickers in ANCA Associated Vasculitis
Authors: Y. Yagiz Ozogul, S. N. Esatoglu, M. Ozogul, O. Kizilkilic, Y. Ozguler, V. Hamuryudan, G. Hatemi
Journal: Annals of the Rheumatic Diseases (2022)

Background Central nervous system (CNS) involvement is rare in ANCA associated vasculitis (AAV). Besides, complications of immunosuppressive (IS) therapy or other conditions that mimic CNS involvement may occur in some AAV patients. Objectives We aimed to assess the clinical, laboratory and imaging features of our AAV patients with CNS involvement and conditions other than CNS involvement that caused neurologic signs and symptoms. Methods We reviewed the charts of our AAV patients with neurologic sign or symptoms and extracted data on their demographics, types of AAV, neurologic symptoms/signs, final diagnoses after neurologic work-up, and their outcome. Results Nineteen AAV patients (13 men, mean age: 46.6±16.6 SD years) with neurologic signs or symptoms were identified. Fifteen patients had GPA, 3 had MPA, and 1 had EGPA. Neurologic symptoms were present at disease onset in 9 patients while they developed within a mean follow up of 36.7 ± 40.1 SD months after AAV diagnosis in the remaining 10. At the time of the occurrence of neurologic symptoms, all patients had active disease (median (IQR) BVAS: 13.8 (9.5-18.5)). Eight patients (42%) also had accompanying peripheral nervous system involvement. Final diagnosis was CNS involvement of AAV in 5 (26%) patients. These were ischemic cerebrovascular accident (CVA) in 2 patients, hemorrhagic CVA in 1 patient, and cranial neuropathy (peripheral facial nerve palsy) in 2 patients. Cranial MRI revealed T2 FLAIR hyperintensities in 2 patients with ischemic CVA. One also had border zone ischemic changes and the other had areas compatible with terminal branch ischemia. Corticosubcortical hematoma in the left parietal lobe and microhemorrhages in the right frontal lobe were observed in a patient with hemorrhagic CVA. Among the 2 patients with facial nerve palsy, cranial MRI was normal in one while the other had nonspecific increased T2 signals in the cerebral cortex. Cranial neuropathy resolved with high dose glucocorticoid (GC) treatment without sequel in 1 patient and regressed with high dose GC and cyclophosphamide (CYC) in the other. One patient with ischemic CVA was lost to follow-up, and the other recovered with high dose GC and CYC treatment without sequela. The patient with hemorrhagic CVA had died. Neurologic symptoms were diagnosed to be due to other AAV manifestations in 6 (32%) patients. These were sinonasal involvement in 3 patients with sensorineural hearing loss (n=2), and blurred vision (n=1); orbital involvement in 2 patients with headache (n=2) and ocular involvement (scleritis) in 1 patient with blurred vision. Neurologic symptoms of these 6 patients recovered with immunosuppressive therapy including high dose GC (n=6), mycophenolate mofetil (n=2), methotrexate (n=2), rituximab (RTX) (n=1), and both CYC and plasmapheresis (n=1). Three patients (16%) had secondary complications affecting the CNS. One patient with seizures had posterior reversible encephalopathy syndrome (PRES) and recovered with CYC and anti-epileptic drugs. The second patient with blurred vision and headache had cerebral venous sinus thrombosis (CVST) and recovered with anticoagulant therapy. The third patient with muscle weakness died due to spondylodiscitis complicated with aortic pseudoaneurysm. In 5 patients (26%), neurologic work-up did not lead to an underlying condition. The presenting symptoms of these patients were transient acute vision loss in 2, numbness of extremities in 1, syncope in 1 and vertigo in 1 patient. Neurologic symptoms resolved after high dose GC and RTX in the patient with vertigo. At the onset of neurologic symptoms, 3 patients were using IS therapy including azathioprine, MMF and CYC in 1 patient each. The fourth patient was off treatment. Neurologic symptoms were transient in these patients, and did not recur during our follow-up of 36, 52, 57, and 120 months. Conclusion CNS involvement appears to be rare in AAV and non-CNS entities including ocular, orbital and sinonasal involvement and complications such as PRES, CVST and infections may mimic CNS involvement in patients with AAV. Disclosure of Interests Yeliz Yagiz Ozogul: None declared, Sinem Nihal Esatoglu Speakers bureau: Sinem Nihal Esatoglu has received honorariums for presentations from UCB Pharma, Roche, Pfizer, and Merck Sharp Dohme, Murat Ozogul: None declared, Osman Kizilkilic: None declared, Yesim Ozguler Speakers bureau: Yesim Ozguler has received honorariums for presentations from UCB Pharma, Novartis, and Pfizer., Vedat Hamuryudan Speakers bureau: Vedat Hamuryudan has served as a speaker for AbbVie, Celgene, Novartis, and UCB Pharma., Grant/research support from: Vedat Hamuryudan has received grant/research support from Celgene., Gulen Hatemi Speakers bureau: Gulen Hatemi has served as a speaker for AbbVie, Celgene, Novartis, and UCB Pharma, Grant/research support from: Gulen Hatemi has received grant/research support from Celgene.